Case report: airway and concurrent hemodynamic management in a neonate with oculo-auriculo-vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine

Paediatr Anaesth. 2012 Sep;22(9):932-4. doi: 10.1111/j.1460-9592.2012.03915.x.


We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured.

Publication types

  • Case Reports

MeSH terms

  • Airway Management
  • Aortic Arch Syndromes / pathology
  • Aortic Arch Syndromes / physiopathology
  • Aortic Arch Syndromes / therapy*
  • Ductus Arteriosus, Patent / complications
  • Ductus Arteriosus, Patent / pathology
  • Female
  • Goldenhar Syndrome / pathology
  • Goldenhar Syndrome / physiopathology
  • Goldenhar Syndrome / therapy*
  • Heart Septal Defects, Ventricular / pathology
  • Heart Septal Defects, Ventricular / physiopathology
  • Heart Septal Defects, Ventricular / therapy*
  • Hemodynamics / physiology
  • Humans
  • Infant, Low Birth Weight
  • Infant, Newborn
  • Intubation, Intratracheal
  • Joint Instability / pathology
  • Joint Instability / physiopathology
  • Joint Instability / therapy*
  • Magnetic Resonance Imaging
  • Scoliosis / pathology
  • Scoliosis / physiopathology
  • Scoliosis / therapy*
  • Spine / pathology
  • Tomography, X-Ray Computed